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39 Cards in this Set
- Front
- Back
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CLL is defined as...
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mature
<20% blasts |
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a lymphoma...
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presents primarily with a tumourous tissue mass
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a leukemia...
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presents primarily with blood & BM involvement
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What defines a population as lymphoid?
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surface antigens
antigen receptors (B or T) gene rearrangement |
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What defines a lymphoid population as neoplastic?
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population of clonal lymphocytes capable of formation of tumorous masses or invasive growth
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What is an alternative definition for neoplastic lymphoid population?
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clone of lymphocyte with aquired genetic lesions confering autonomy from normal regulatory controls
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What is the demographics for CLL/SLL?
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most common leukemia of US
M:F 2:1 >50 yo |
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Charactersitics of CLL
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asymptomatic
hypogammaglobulinemia!!! autoimmune phenomena BM involved |
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Immunophenotype of CLL is
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CD 5+
CD 19 + pan B Ag CD 10 - CALLA CD 23 + FMC7 - |
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What are the major prognostic factors for CLL?
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stage
karyotype mutational status Zap 70 expression |
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IgH genes in B cells somatic hypermutation
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normal B cells get many random pt mutations in Ig variable region during passage through germinal centers to generate Ab diversisty
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How are IgH gene Bcell lymphomas categorized?
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pre germinal center
germinal center post germinal genter |
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pre germinal center b-cells
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no somatic hypermutation
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Germinal center B-cells
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ongoing somatic hypermutation
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Post germinal center Bcells
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stable pattern of somatic hypermutation
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What is the main mutational status change in CLL?
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IgV
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what are the characteristics of unmutated IgV CLL
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aggressive
poor prognosis |
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What does Igv coorelate with?
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Zap 70 expression
Cd 38 |
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What are the transformations seen in CLL?
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prolymphocytoid
Ricthers syndrome |
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What is prolymphocytoid transformation?
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gradual accumulation of prolymphocytes in blood
clonal evlolution more aggressive |
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What is Ricther's syndrome?
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pleomorphic large B cell lymphoma
abrupt change poor prognosis various genetic lesions |
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What is the pt profile for hairy cell leukemia?
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middle aged guy iwth cytopenias & a big spleen
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What is the main immunophenotype for hairy cell leukemia?
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CD 103 +
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What are the other immunophenotypes fdor hairy cell leukemaia?
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CD 19 +
CD 20 + CD 5 - CD 10 - Co expression CD 11 & 22 |
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How do you treat hairy cell leukemia?
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2-CDA
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What is large granular lymphycyte leukemia?
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expansion of clonal CD 8+ T cells
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What is the patient profile for large granular lymphocyte leukemia?
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~60
male slightly more than female |
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What are sx of large granular lymphocyte leukemia?
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splenomegaly 50%
rare LA RA in 30% pts NEUTROPENIA |
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Prognosis of large granular lymphocytic leukemia?
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stable clinical course
lacks destructive growth |
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What is sezary syndrome?
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leukemic form of mycosis fungoides (indolent cutaneous Tcell lymphoma)
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What are sx in Sezary syndrome?
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erythroderma
LA peripheral blood lymphocytosis highly aggressive |
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What is the patient profile for sezary syndrome?
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M>F
~50 |
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What do cells of sezary syndrome look like?
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ceribriform cells - grooves, clefts & infoldings making the cell look like a brain
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What is the immunophenotype for sezary syndrome?
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CD4+
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What is adult T-cell leukemia assoicated with?
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HTLV-1
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Where do you see HTLV-1?
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SW Japan & caribbean
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What are the sx in adult T-cell leukemia?
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LA, hepatosplenomegaly, skin infiltration, hypercalcemia
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What do the cells of adult T-cell leukemia look like?
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deep nucelar clefts - flower
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What is the immunophenotype for adult t-cell leukemia?
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CD 4+ (like SS)
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